Department of Paediatric Surgery | Cairo University Specialized Paediatric Hospital (CUSPH) & Cairo University Children's Hospital (CUCH) (email)
Congenital adrenal hyperplasia in females leads to virilization of the external genitalia and an anomalous genitourinary tract, with a termination of the urethra and vagina into the persistent urogenital sinus. Surgical repair during infancy is indicated to raise a female child with external genitalia matching her gender and to avoid psychosocial and genitourinary complications. Earlier surgery is believed to be easier and of better results. Twenty five female patients with virilized external genitalia and persistent urogenital sinus due to congenital adrenal hyperplasia were studied. Interpretation of the genitography and cystoscopy was analyzed and compared to the operative findings to assess their accuracy and predictability. Sensitivity of cystoscopy and genitography is comparable (78.5% and 73% respectively), but cystoscopy has a higher specificity than genitography (91% and 83% respectively). Both investigations had a similar predictivity and were able to reveal the internal anatomy and operative difficulty in 56% of cases. In 12% both investigations had a similar predictivity, but failed to expect the operative difficulty. Cystoscopy was superior to genitography in 24%, but surprisingly genitography was more predictive in 8%. Neither genitography nor cystoscopy were independently predictive of the internal anatomy and intraoperative findings or difficulty of the studied cases. We therefore recommend doing both investigations as a routine preoperative workup. The benefit is to assess these cases properly and support decision making and choice of surgery.
Key words: DSD, Feminizing Genitoplasty, Cystoscopy, Genitography.
Kasr El Aini Journal of Surgery. 01/2014; 15(1):21-25.